Autoimmune Hepatitis Presenting with Acute Liver Failure: INR ≥ 2 and Hepatic Encephalopathy

Clinical Scenario

This protocol addresses autoimmune hepatitis (AIH) when it presents with, or progresses to, acute liver failure — defined by an INR of 2 or greater alongside hepatic encephalopathy arising within 26 weeks of illness onset, in a patient without previously recognised liver disease.

Acute Liver Failure INR ≥ 2 Hepatic Encephalopathy No prior liver disease

Defining Features

The combination of INR ≥ 2 and hepatic encephalopathy within 26 weeks of onset — in the absence of a previously known liver condition — identifies AIH with acute liver failure (ALF). This subset carries a distinct clinical course that separates it from acute severe AIH without encephalopathy, and it calls for a different management pathway.

Management Approach

For patients with AIH and ALF, current evidence points to a specific, time-sensitive intervention distinct from the approach used in acute severe AIH without encephalopathy. The full structured protocol details the recommended pathway and the criteria that guide it.

Complete regimen details, sequencing, and decision criteria are available in the full protocol below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1002/hep.31065

ALF INR ≥ 2; hepatic encephalopathy within 26 weeks of onset of illness; no previously recognized liver disease

Patients with acute severe AIH should receive a treatment trial with prednisone or prednisolone alone, whereas patients with AIH and ALF should be evaluated directly for LT.

Patients with acute severe AIH should receive predniso(lo)ne followed by LT if no improvement within 2 weeks, whereas patients with AIH and ALF should be evaluated directly for LT.

Hepatic encephalopathy at presentation defines AIH with ALF, and LT is more likely to improve survival than protracted glucocorticoid treatment.

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